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Ovarian diseases. Dr Ismaiel Abu Mahfouz. Benign ovarian diseases. Ovarian Cysts. Prevalence 4% of women are admitted to hospital with an ovarian cyst / complication by the age of 65 years 25% of adnexal torsions occur in children 90% of all ovarian cysts are benign
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Ovarian diseases Dr Ismaiel Abu Mahfouz
Ovarian Cysts Prevalence • 4% of women are admitted to hospital with an ovarian cyst / complication by the age of 65 years • 25% of adnexal torsions occur in children • 90% of all ovarian cysts are benign • Risk of Ca in an ovarian cyst in a woman of: • Reproductive age: 0.4–0.8/100 000 • Age 60–80 years : 60/100 000
Ovarian cyst events / complications Rupture • Asymptomatic / acute abdominal pain • May follow sexual intercourse or physical activity • Severity of pain depends on the type of fluid “Serous or mucinous/ sebaceous material/Blood” Haemorrhage into a cyst: • Pain of variable degree. Usually mid cycle Torsion • Moderate-severe pain & of sudden onset • Associated nausea and vomiting Infection • Pain, fever, peritoneal irritation ??PID
Clinical evaluation History • History of endometriosis/ PID/ known ovarian cysts • Bowel / urinary symptoms • Anticoagulants • Progesterone only pills: develop recurrent ovarian cysts • Pain may be referred down the cutaneous distribution of the Obturator nerve (inner thigh down to the knee) Examination • +/- low-grade fever. BP,PR: usually stable • Abdominal tenderness • Cervical excitation on vaginal examination
Investigations • Pregnancy test • Urinalysis and culture • Full blood count, urea and electrolytes • ? Coagulation screen • Genital swabs for infection if PID is suspected • CA-125 : Not as a routine • Ultrasound examination • Doppler blood flow of the cyst: Findings are variable and not diagnostic
Ovarian cyst with typical mixed internal echoes suggestive of blood
Adnexal torsion Enlarged ovary / adnexal mass Free fluid if associated with rupture Doppler sonography is not helpful in the diagnosis
D.Dx In case of ovarian cyst complication, consider • Ectopic pregnancy • Pelvic inflammatory disease • Pelvic abscess • Fibroid degeneration • Appendicitis • Complications of diverticular disease • Urinary tract infection • Urinary calculi • Renal colic
Management • Expectant Mx: Haemorrhagic cysts and cyst rupture Analgesia and observation • Repeat scan 6 wks • Surgery: Laparoscopy /Laparoscopy if: • Haemodynamic compromise • Diagnostic uncertainty or likelihood of torsion • No relief of symptoms within 48 hours of presentation • Consider COCPs for cyst formers
Special situation Ovarian cysts in pregnancy • Most common: Dermoid cysts (50%) then cystadenomas • < 5% require intervention • Conservative management is appropriate • Indications for intervention: • Symptomatic relief • Suspicion of malignancy
Special situation Ovarian tumours in children • Ovarian ca represent 1.5% of childhood ca • Most ovarian tumours are benign • Types: • Most common: Epithelial cysts and teratoma • Most common ca: Germ cell tumours • Most common complication: Torsion (33% of cases)
Malignant disease of the ovary Ca ovary • The second most common gynae ca after uterine ca • 5th most common ca in women after breast, bowel, lung and uterine ca • The majority of ovarian ca are epithelial
Types of Ovarian cysts / tumors Functional • Follicular cyst • Corpus luteum cyst • Theca lutein cyst Inflammatory • Tubo-ovarian abscess Benign tumours/cysts • Endometriotic cyst • Brenner tumour • Benign teratoma • Fibroma Malignant /malignant potential • Epithelia ovarian ca • Malignant teratoma • Endometrioid carcinoma • Dysgerminoma • Secondary ovarian tumor • Cystadenoma, cystadenocarcinoma • Granulosa cell tumor • Arrhenoblastoma • Theca cell tumor
Classification of ovarian tumours • Ovarian tumors classified according to their origin, biological behavior or clinical manifestations • WHO Classification: • Epithelial • Sex cord stromal • Germ cell
Epithelial tumours(80 - 90%) Serous Tumors • Benign/Borderline/ ca • Can be bilateral • Psammoma bodies • BRCA 1 mutations Mucinous Tumors • Benign/Borderline/ ca • Pseudomyxoma peritonei • RT / CT resistant Endometroid Tumors • Malignant • ? Endometriosis • Ass. with endometrial ca Clear Cell (Mesonephroid) • Benign/Borderline/ ca • Worst prognosis Transitional cell (Brenner) • Usually benign Mixed epithelial tumours Undifferentiated & unclassified
Germ Cell Tumours (10-15%) Dysgerminoma • Most commonly malignant • Abnormal gonads/Turner • Bilateral • LDH • Chemo/radiosensitive Endodermal Sinus Tumors (Yolk Sac Tumors) • Young children < 4yrs • 3rddecade • Schiller-Duval bodies • AFP Choriocarcnoma • Malignant • Cyto-& syncitiotrophoblast • B-HCG Teratomas • Immature- can get malignant • Mature • Solid • Cystic • Monodermal & highly specialized • Struma ovarii • Carcinoid • Struma Ovarii & Carcinoma • Mixed forms GONADOBLASTOMA • Pure • Mixed with Dysgerminoma or other Form Germ Cell Tumors
Sex Cord Stromal Tumours(5-10%) Granulosa-Stromal Cell Tumors • Granulosa cell • Any age • Inhibin A/B or Estradiol • Precocious puberty • Microscopic: Call-Exner bodies • Tumours in the Thecoma-fibroma group Androblastomas • Sertoli-Leydig Cell Tumors • Well /Intermediate/Poor differentiated • Secretes androgen Fibromas • Associated with ascites & hydrothorax “Meigs syndrome”
Krukenberg tumour • Secondary Ca of the ovary • Metastasized classically from GIT and breast • 80%: bilateral ovarian involvement • “ Signet ring cells”
Ovarian tumours • Primary ovarian ca commonly: 40-60 yrs • Teratomas and Sex Cord: mostly before puberty • Borderline malignant: 30-50 yrs Ovarian ca; a silent killer • Asymptomatic in early stages • 75% diagnosed with advanced stage disease • Overall 5-year survival rate: 35% • Most common cause of death from gynae ca in UK
Ovarian ca; risk factors Ovarian ca • Most cases of EOC are sporadic • The aetiology is unknown • Most significant risk factor is genetic predisposition Ovarian ca, a challenging disease • Natural history not well understood • No well-defined precursor lesion • Length of time from localised tumor to dissemination is unknown • No effective screening method for early detection yet
Risk factors: Heredity • 10% of Epithelial ca cases are familial • Familial syndromes: • Familial breast-ovarian cancer syndrome (BRCA I+II) • Cancer family syndrome (Lynch syndrome = HNPCC) • Account for 90% of familial ovarian ca
Additional Risk Factors • Age • Rare <30 • Peak ≥ 60yrs • Reproductive history • Early menarche • Multiparity or age >30 at first child-bearing • Late menopause • Fertility drugs • Personal history of breast cancer • Talcum powder
Protective factors • Multiparity: First pregnancy before age of 30 • Oral contraceptives: 5 years of use decreases risk by 50% • Tubal ligation • Hysterectomy • Lactation • Bilateral oophrectomy
Diagnostic approachHistory • Abdominal bloating, increased girth, pressure • Unusual fatigue • GIT: nausea, indigestion, gas, constipation, diarrhea • Urinary frequency or incontinence • Unexplained weight loss or gain • Shortness of breath Germ cell tumours • Often present more acutely & at an earlier stage • Typically: • Rapidly enlarging abdominal/pelvic mass • Acute severe lower abdominal pain due to tumour rupture, haemorrhage or torsion
Diagnostic approach Examination • Abdominal / pelvic: pelvic masses, ascites, hepatomegaly • Chest: Pleural effusions, palpable lymph nodes Imaging • TA & TV scans: Detection of masses and its characters • CT scan (Abdomen / chest): Assess spread to LN, pelvic & abdominal structures • MRI: Best to distinguish malignant / benign tumors Bloods: CBC, KFT, LFT, tumour marker
Diagnostic approachTumour markers • Serous tumours: CA 125 • Mucinous: CA 19-9 • Granulosa: Inhibin • Endodermal sinus: AFP • Choriocarcinoma: HCG • Dysgerminoma: LDH, Alkaline phosphatase
Diagnostic approachRisk of malignancy index (RMI) RMI: • Gives an estimate of the risk of ovarian ca for women with adnexal masses • Calculated using • Ultrasound findings (U) • Menopausal status (M) • CA-125 value (serum levels >30U/ml abnormal)
RMI RMI = U x M x CA125 Ultrasound findings (U) “Scored 1 point for each” • Multi-locular cyst • Evidence of solid areas • Evidence of metastases • Presence of ascites • Bilateral Lesions U: • U = 0 (U/S score of 0) • U = 1 (U/S score of 1) • U = 3 (U/S score of 2 – 5) Menopausal status • Postmenopausal status is graded M = 3 • Pre-menopausal status is graded M = 1 Ca-125
Ultrasound • Both TA and TV ( TVS has better resolution) • Major limitations • Poor PPV in asymptomatic women • Inability to detect ca when ovaries are normal size • Allows earlier stage detection
Benign vs Malignant Tumors “ Ultrasound & Doppler” Benign Malignant More likely bilateral Multilocular Thick walls Papillae present Mixed echogenicity due to solid areas Greater Angiogenesis and Blood Flow • More likely unilateral • Unilocular • Thin-walled • No papillae • No solid areas
Spread of Ovarian malignancies • Direct seeding: To peritoneum, omentum, tubes, ureters • Lymphatics:To para-aortic nodes, umbilicus, diaphragm • Bloodstream:To lower vagina and in the case of sarcomas and Teratomas to the lungs and else where • Direct spread:To any neighboring organ or tissue
Ovarian ca: Staging FIGO ovarian cancer staging: 2014 update
Ovarian ca: FIGO Grading Epithelialtumours of the ovary are also sub-classified by histological grading proportional to prognosis. • Gx : Grade cannot be assessed • G1 : Well differentiated • G2 : Moderately differentiated • G3 : Poorly differentiated
Treatment Options • Surgery • Chemotherapy • Radiotherapy
Standard treatment for ca ovary Surgery Chemotherapy • Platinum • Taxol
Types of Surgery Aimof surgery • Optimal cytoreduction: maximum residual tumour deposits no more than 1 cm • May consider fertility preserving procedure should that be medically possible Types of surgery • TAH+BSO • Unilateral salpingoophrectomy (if fertility has to be preserved) • Cytoreductive or “debulking” • Peritoneal metastasis reduction • “Second look” laparotomy
Chemotherapy (CT) • Ovarian ca is a chemo-sensitive • Advanced disease “ has progressed beyond the ovaries, stage 1c & above; require both surgery and CT Types of chemotherapy • Adjuvant: CT following surgery • Combination: Several agents given simultaneously to enhance their effectiveness • Neo-adjuvant: CT prior to surgery where Dx has been established by cytology of ascitic fluid or histology of a tissue biopsy
Chemotherapeutic Agents • Alkalyting agents: Cyclophosphamide, Cisplatin, Carboplatin, Melphalan • Plant alkaloids: Paclitaxel, Vincristine, Etoposide • Anticancer antibiotics: Bleomycin, doxorubicin • Antimetabolites: Fluorouracil, Gemcitabine
Side effects of chemotherapy • Nausea and vomiting • Fatigue • Oral ulcerations • Ototoxicity (cisplatin): hearing loss, tinnitus • Peripheral neuritis • Nephrotoxicity • Myelosuppression • Pulmonary toxicity (bleomycin). Any new-onset cough/shortness of breath should be investigated urgently to exclude pneumonitis or fibrosis.
Follow up after primary treatment Follow up • Provide reassurance • Assess for early recurrence prior to the onset of significant symptoms Follow up • Clinical • CA-125 • MRI